30 closest matches · ranked by relevance
Bile Acid Synthesis Defect · Inborn Error of Bile Acid Metabolism · Inborn Error of Bile Acid Conjugation
Primary: Conjugated Cholic Acid (GCA) for the Treatment of Inborn Errors in Bile Acid Synthesis Involving Side-chain Conjugation. — 5 Participants
Infantile Refsum's Disease · Zellweger Syndrome · Adrenoleukodystrophy
Primary: Number of Participants With Excretion of Atypical Bile Acids in Urine by Category — 10; 51; 11; 9 Participants
Bile Acid Synthesis Defect
Primary: Change in Atypical Urinary Bile Acid Excretion by FAB-MS (Fast-Atom-Bombardment Ionization-Mass Spectrometry) — 25; 6; 10; 10 Participants
Smith-Lemli-Opitz Syndrome
Primary: Change in Plasma Cholesterol — 37.8 change (%) compared to baseline — p=0.011
Bile Acid Malabsorption · Cholelithiasis
Primary: Change in Stimulated Fibroblast Growth Factor 19 (FGF19) From Baseline Before Versus Post Cholecystectomy — 81; 186 pg per mL — p=0.03
Primary Bile Acid Malabsorption · Secondary Bile Acid Malabsorption · Chronic Diarrhoea
Primary: Changes in Fasting FGF19 — 71; 25; 130 percentage increase of baseline — p=0.007
Obesity
Primary: Change in 48h-fecal Bile Acid Excretion (μmol/L) Between Baseline and High Fat Diet — 543; 344 (μmol/L)/48 hour
Inborn Errors of Bile Acid Synthesis
Primary: Serum Transaminases — 31.4; 30.9; 62.7; 65.0 U/L
Type 2 Diabetes Mellitus · Obese · Overweight
Primary: Change in Postprandial Glucose — -2803; 629 mmol/6hr — p=<0.05
Smith-Lemli-Opitz Syndrome
Primary: Number of Responders — 18 Participants
Gastroesophageal Reflux Disease
Primary: Protection Against DNA Damage by UDCA — 1.068; .82 Relative Densitometry Units
Progressive Multiple Sclerosis
Primary: Number of Participants With at Least One Treatment-related Adverse Event — 10; 7 Participants
Obesity · Healthy
Primary: Glucagon-Like Peptide-1 (GLP-1) — 18; 17 Participants
Hepatic Steatosis
Primary: Hepatic Triglyceride (%) — 15.9; 14.8 Fat/Fat+Water (%) — p=0.42
Chronic Diarrhea · Irritable Bowel Syndrome With Diarrhea · Bile Acid Malabsorption
Primary: Fasting Serum C4 Levels — 2.2; 50.4 ng/ML
Diarrhea Predominant Irritable Bowel Syndrome
Primary: Change in Total 48 Hour Fecal Bile Acids (BA) From Baseline in Response to Treatment With Colesevelam — 1662; 3496 uM — p=0.012
Cholestasis · Primary Sclerosing Cholangitis · Primary Biliary Cirrhosis
Primary: Percentage of Participants in Whom we Could Quantify Hepatic Transport of 11C-CSar — 100; 100 percentage of participants
Familial Partial Lipodystrophy
Primary: Change in the Liver Triglycerides (TG). — 13.2; 6.4; 12.3; 10.6 percentage of liver triglycerides
Peroxisome Biogenesis Disorders
Primary: Peroxisome Biochemical Functions as Measured by Plasma Very Long Chain Fatty Acid — 0.180; 0.188 ratio
Chronic Diarrhea · Irritable Bowel Syndrome With Diarrhea · Bile Acid Malabsorption
Primary: Total Fecal Bile Acid (BA) Excretion — 10.7; 3.6 μmoles/g — p=0.001
Biliary Atresia
Primary: Number of Patients With Biliary Atresia (BA) Achieving Total Serum Bile Acids Less Than or Equal to 10 *U*Mol/L Within 24 Weeks of Kasai Portoenterostomy (KP) — 0…
Constipation-predominant Irritable Bowel Syndrome
Primary: Colonic Geometric Center at 24 Hours (GC24) — 3.1; 3.5; 2.2 units on a scale
Healthy Volunteers
Primary: Energy Expenditure — 1.37; 1.40; 1.28; 1.27 kcal/min — p=0.0001
Primary Bile Acid Diarrhea
Primary: Number of Patients Reported With Adverse Events , Serious Adverse Events and Death. — 9; 14; 0; 4 count of participants
Functional Constipation
Primary: Colonic Transit at 24 Hours — 2.68; 3.12; 1.93 units on a scale — p=0.059
Smith-Lemli-Opitz Syndrome
Primary: Serum Cholesterol to Total Sterol Ratio — 90.82; 93.99 percent total cholesterol — p=0.002
Irritable Bowel Syndrome With Diarrhea
Primary: Change From Baseline in Average Bristol Stool Form Scale (BSFS) Score Over 4 Weeks of Treatment Period — 5.89; 5.34; -1.25; -1.09 score on a scale
Insulin Resistance · Diabetes · Obesity
Primary: Body Composition — 39; 39; 37; 39 percentage
Urea Cycle Disorder
Primary: Liver Stiffness as Measured by Shear Wave Elastography — 13; 14 Participants
Primary Biliary Cirrhosis
Primary: Absolute Change From Baseline In High-density Lipoprotein (HDL) Cholesterol Concentration — -0.38 mmol/L